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Biomedical subjects

M Slim

Publications and source records attributed to M Slim.

At least 19 recordsLinked to original sources
PubMed1997

Prenatally diagnosed neuroblastoma.

BACKGROUND: Prenatally diagnosed neuroblastomas have been reported in increasing numbers over the past several years, and there are now a few reviews based on up to 21 cases. The purpose of this article is to review the clinical and biologic features of prenatally diagnosed neuroblastoma based on a review of 55 cases. METHODS: A review was conducted of 3 cases seen at the study institution and 52 other cases reported thus far in the literature. RESULTS: Prenatal diagnosis was made usually after 32 weeks of gestation. Approximately 93% of the tumors were adrenal in origin, and 44% of these were cystic. Thirty-seven patients (67%) had Stage I disease, 12 (22%) had Stage IV-S disease, and only 3 (5%) had Stage IV disease. The DNA index was favorable (> 1) in 14 of 16 patients studied. None of these 16 patients studied had amplification of the N-myc oncogene. Catecholamines were elevated in only 33% of the patients. The liver was the most common site of dissemination, which was observed in 25% of patients; bone involvement was not observed in any patient. Ultrasonography failed to detect existing hepatic metastasis in three patients. Primary surgical resection was performed in 47 patients (85%). Chemotherapy was given to five patients and radiotherapy to three. Of the 50 patients for whom follow-up information was available, 45 (90%) were alive at a range of 2-120 months from diagnosis. CONCLUSIONS: Prenatally diagnosed neuroblastomas are predominantly adrenal in origin and frequently cystic. The liver is the most common site of dissemination and bone involvement is notably absent. The vast majority of these infants have a favorable stage of disease (I, II, and IV-S) and favorable biologic features, and consequently have an excellent prognosis. Although surgery alone is curative for most patients, a period of observation may avoid surgery in some individuals who may achieve spontaneous regression.

Adrenal Gland Neoplasms↗
PubMed1996

Langerhans cell histiocytosis associated with partial DiGeorge syndrome in a newborn.

PURPOSE: We report the unrecognized association of Langerhans cell histiocytosis (LCH) with partial DiGeorge syndrome. PATIENT AND METHODS: A 7-week-old infant with endocrine and immunologic characteristics of DiGeorge syndrome displayed multisystem involvement of Letterer-Siwe disease at birth. RESULTS: Despite vigorous medical support and chemotherapy, she died at 9 months of age with multisystem failure. CONCLUSIONS: This case supports the role of the thymus n the pathogenesis of LCH.

DiGeorge Syndrome↗
PubMed1996

Gastric ganglioneuroblastoma: a rare finding in an infant with multifocal ganglioneuroblastoma.

PURPOSE: This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement. PATIENT: The patient had a right cervical tumor, a left posterior mediastinal tumor, bilateral adrenal tumors, and bony and bone marrow metastases. The tumor cells were diploid and lacked N-myc gene amplification. The gastric involvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy. RESULTS: Our patient achieved only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currently has stable, unresectable disease with elevated catecholamines. CONCLUSIONS: Multifocal ganglioneuroblastomas may arise from either neuroblastic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. Patients with multifocal ganglioneuroblastomas warrant meticulous radiographic and surgical evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.

Female↗
PubMed1989

Combined occurrence of chyloperitoneum and chylothorax after surgery and chemotherapy for Wilms' tumor.

Chyloperitoneum is an extremely rare complication of abdominal surgery in children and a combined occurrence of chylothorax and chyloperitoneum after abdominal surgery has never been reported in children. Chylous ascites usually occurs as a result of operative trauma to the thoracic duct, cisterna chyli, or its tributaries. About one third of all patients with chylous ascites after retroperitoneal lymph node dissection also develop secondary chylothorax. Diaphragmatic defects have been shown to be responsible for the occurrence of chylothorax secondary to chyloperitoneum. Congenital diaphragmatic weakness may result in evagination of the peritoneum causing diaphragmatic blebs, the rupture of which results in the movement of the peritoneal fluid into the pleural cavity. In the authors' patient, the rent in the diaphragm that occurred during surgery was probably responsible for the chylothorax. The role of chemotherapy, if any, in the pathophysiology of this complication is unknown. Total parenteral nutrition (TPN) is a simple and effective treatment for postoperative chylous effusions. Surgical treatments such as abdominal exploration for the repair of leaking lymphatics and peritoneovenous shunt should be reserved for patients who fail TPN.

Chylothorax↗
PubMed1988

Wandering intravascular missiles: report of five cases from the Lebanon war.

From 1980 to 1986, during the Lebanon war, five patients with missile embolization were seen at the American University of Beirut Medical Center. Three had entry in the heart or thoracic aorta with peripheral embolization, and two had entry in the internal carotid artery and inferior vena cava with embolization to the middle cerebral artery and heart, respectively. Embolization was suspected when, in the absence of an exit, routine x-ray films showed the missile in a distant location. Angiography and echocardiography confirmed the diagnosis. Peripheral arterial emboli were extracted while cerebral and venous emboli were kept, as they caused transient symptoms and remained silent.

Adolescent↗
PubMed1986

Neonatal appendicitis: case report and a revised review of the English literature.

A case of perforated neonatal appendicitis in a surviving premature infant is presented. An up-dated review of the English literature on this subject is included. Of 111 cases with adequate documentation, 29 neonates had the inflamed appendix located within the inguinal hernial sac and survived after operation. In contrast only 24 (29%) of the remaining 82 patients with intra-abdominal acute appendicitis survived. The operative mortality rate in this review since 1901 with and without appendiceal perforation was 61% and 41% respectively. The diagnosis of acute appendicitis since 1976 was made at laparotomy in all patients except one, whereas it was made at necropsy in 57% of the cases of abdominal appendicitis that were reported previously.

Appendectomy↗
PubMed1985

Tetralogy of Fallot with pulmonary atresia in siblings.

We present two sisters with tetralogy of Fallot and pulmonary valve atresia. Both had identical anatomical findings as seen at cardiac catheterization and angiography and verified operatively, with, in particular, identical bronchial circulation and pulmonary valve structure. The parents are first cousins and there is no history of other affected relatives. We suggest that this is a specific, recessively inherited type of tetralogy of Fallot.

Child↗
PubMed1985

Spinal cord compression in neuroblastoma.

Twelve of 80 patients suffering from neuroblastoma who were treated during a 21-year period had intraspinal involvement. Mediastinal tumors have a greater tendency to extend to the spinal canal; however, distant spread of the tumor is rare in patients presenting with intraspinal extension. Patients with intraspinal extension also survive longer than those without. Other factors affecting survival are age, stage of disease, duration of neurological symptoms, degree of histologic differentiation, and mode of therapy. In the absence of osseous metastasis, total excision of the primary lesion and its intraspinal components is usually followed by a favorable outcome; residual neurological deficits among survivors, however, are relatively common.

Child↗
PubMed1984

Ehlers-Danlos syndrome type IV D: an autosomal recessive disorder.

Two siblings born to consanguineous parents are reported with typical clinical features of the Ehlers-Danlos syndrome type IV. However, their cultured skin fibroblasts synthesize and secrete procollagen type III in normal amounts and proportions. This is probably a new form of the Ehlers-Danlos syndrome with autosomal recessive inheritance classified as Ehlers-Danlos syndrome type IV D.

Biopsy↗
PubMed1982

One-lung ventilation of children during surgical excision of hydatid cysts of the lung.

One-lung ventilation was used in 13 children, undergoing surgical excision of pulmonary hydatid cysts. Five had right-sided pulmonary cysts and eight had left cysts. An ordinary cuffed tracheal tube was introduced to the main bronchus of the healthy side. One-lung ventilation using 1-2% halothane in oxygen produced PaO2 values of 12.6-33.3 KPa and PaCO2 values of 4-6kPa. After operation there was a high frequency of right upper lobe collapse in cases of selective right bronchial intubation, but this cleared completely within 3-4 days. The technique proved to be a simple and effective method of isolating and ventilating the healthy lung.

Adolescent↗
PubMed1979

Adrenal cysts: pathogenesis and histological identification with a report of 6 cases.

Because of their rarity cysts of the adrenal gland are often misinterpreted and misdiagnosed. Six cases of surgically removed adrenal cysts are presented, including 3 endothelial cysts, 2 epithelial retention cysts and 1 pseudocyst. Evidence is presented that epithelial retention cysts could and do develop in the human adrenal cortex and are not theoretically impossible as propagated in the literature. We also emphasize the importance of evaluating the surrounding fibrous and adrenal tissue in the proper identification of these cysts, a point not sufficiently stressed previously.

Adrenal Gland Diseases↗